Pain in the person with sickle cell disease, because most often it will re-offend , should be treated quickly and effectively .

Repeated pain, by the way they are experienced , have an impact on the psychological and physical development of the child and on his way to feel the painful crises that will occur again and again.

An incurable disease

Currently sickle cell disease is incurable, but adequate medical care can prevent the effects.

  • Drink plenty of liquids
  • Tylenol (acetaminophen)
  • Oral and intravenous drug therapy
  • Prophylactic penicillin taken daily to prevent infections in children from 2 months to 5 years
  • Blood transfusions
  • Hydroxyurea treatment
  • Daily intake of folic acid supplements
  • Bone marrow transplants

DETECTION OF SICKLE CELL ANEMIA

Most states in the US and some Canadian hospitals are now automatically take a simple blood test to all newborns at risk. This test is done simultaneously and with the same blood samples as other routine tests for newborns. The hemoglobin electrophoresis ” hemoglobin electrophoresis ” is the most used. In Ontario, it already carries prenatal routine screening. If the results show the presence of the sickle hemoglobin , a second blood test will be administered to confirm the diagnosis. These tests can also show if the child is a carrier of the disease gene.

NEW DRUGS HYDROXYUREA

Hydroxyurea is a drug (capsule) that was originally used to treat some forms of blood cancer (Leukemia) and blood disorders that can later turn into Leukemia. It has been used for over 15 years to treat Sickle Cell Disease.

Hydroxyurea works in a few different ways, but the main way is to increase your body’s production of Fetal (baby) Hemoglobin. We know that this then protects you against Sickle Cell Disease. It is a safe drug and there is no evidence to suggest increases the risk of cancer in patients with Sickle Cell Disease. Like all drugs, it does have some side effects, and you need regular blood test monitoring. Also, unlike pain killers which you take when required, Hydroxyurea must be taken every day to be effective.

Hydroxyurea is the only drug therapy available for Sickle Cell Disease and has been shown to prolong survival, reduce pain episodes and reduce some of the complications. Unless you have been told by a specialist that you have very mild Sickle Cell Disease, you should consider going on the drug.

Why am I being offered Hydroxyurea? ?

You are most likely to be offered Hydroxyurea because you have lots of painful crisis or Acute Chest Syndrome. It may also be offered if there is concern about damage to any of your body organs from the Sickle Cell Disease, or as an alternative to blood transfusion treatment.

BLOOD TRANSFUSION

Blood transfusions in Sickle Cell Disease can be carried out in 1 of 3 ways. The easiest way is to simply transfuse units of blood, or a “top-up transfusion”. This improves the supply of oxygen to your body. The most complex way of receiving blood is by “automated red cell exchange” (erythrocytopheresis). This involves hooking you up to a machine that automatically filters of the sickled Red Blood Cells and replaces it with new, normal Red Blood Cells. The alternative to these, is a “partial manual exchange transfusion”, whereby a nurse will remove 2 units of blood and then give you a 2 unit transfusion.

These 3 methods all have advantages and disadvantages and are suitable for different reasons.

Transfusions are most commonly given for patients who have had a stroke or are found to be at high risk of having a stroke. It can also be given for a severe crisis, such as Acute Chest Syndrome or if you are very sick and need to be cared for in Intensive Care. You may need a blood transfusion if you are very anemic (aplastic crisis), which is usually due to a viral infection (cough or cold). It may also be appropriate to give you a transfusion if you are pregnant and there are complications, or if you are planning on having major surgery.

Phenotypic blood

Phenotypic matched blood means that the blood has been matched to your blood group as closely as possible. This reduces the chance of having a transfusion reaction and of developing an Antibody to the blood. However, because most blood donors are from ethnic backgrounds different to Sickle Cell Disease patients, there is a limited supply of suitable blood.

How can I help increase phenotypical blood reserve ?

The Canadian Blood Service (CBS) and Hema-Quebec would like more blood donors from the ethnic minorities. If you have friends or family who want to help people with Sickle Cell Disease, becoming a blood donor, is a very practical, valuable and worthwhile way to help. If you would like to arrange a blood donor drive or session, the Hema- Quebec and the CBS can help with this. You cannot donate blood if you have Sickle C.

COMPREHENSIVE CARE

Sickle cell disease is a life-long condition whose symptoms and complications have considerable variability among the affected individuals – as well as during different times in the life of a particular affected individual. Currently, successful hematopoietic stem cell transplantation is the only cure but is only available for a small minority of individuals due to the lack of suitable donors and other reasons.

For infants with sickle cell disease, serious health complications can arise within a few months after birth such as life threatening infections, vasoocclusive crises, etc., which makes early involvement by expert medical teams essential. Their parents need to be educated to anticipate complications so that prompt and appropriate actions can be taken to ensure the safety and optimal development of these children. Early adolescence is the optimal time for the individuals to learn about the detail nature of their own condition, to be aware of how to stay healthy and to know how to advocate for themselves. The nurturing of this knowledge is critical in addition to their regular scheduled clinic appointments. Adolescence and young adult period can be challenging times as the person with sickle cell disease achieve greater independence in the midst of peer pressure in schools and in their vocations. The building of a healthy self-image with motivation to maintain good health is paramount. Aging inevitably brings with it concerns with sickle cell related premature organ damage on top of the normal wear and tear processes. Recent research in this area together with the help of expert teams is needed to deal with the various concerns. Throughout these critical stages in life, persons with sickle cell disease have distinct physical and psychosocial needs that are different from the unaffected population at large. They will require comprehensive health care through expert medical centers that can address their diverse health needs.

So what is comprehensive care ?

Comprehensive care deals not only with management of sickle cell disease, but also places a great deal of importance in the prevention of its complications and the creation of a healthy life outcome.  It addresses both the physical and emotional health at all stages of life, and provides care to the persons with sickle cell disease in the context of his (her) family, life events and environment.

The optimal core comprehensive care team should include hematologists and specialty nurses with expertise and interest in sickle cell disease. Medical social workers and psychologists are needed to assist with psychosocial challenges.  The support of medical experts such as Emergency room physicians, Pain management physicians, Intensivists, Pediatricians, Internists, Laboratory and Transfusion medicine physicians, Medical geneticists, Obstetricians are indispensible at different times.  It will be importance for the core medical team to keep these supporting physicians updated with the newest knowledge in the field, and to ensure close communication when a patient is seen.

The expert medical center should have 24/7 coverage to attend to patients with acute medical problems, or be able to offer real-time medical advice at a distance should patients show up their local hospitals.  In addition, the provision of services for patients and families, including education, counseling, support groups, advocacy, and other special programs will help to improve the quality of life of patients and their families.

Until a cure is achieved for everyone affected with sickle cell disease, the offer of optimal comprehensive care to these individuals will enable them to have the best clinical outcome, and a good quality of life.


Acknowledgement : Some of the information on this page was provided by:
John K. Wu, MBBS, MSc, FRCP(C)
British Columbia Children’s Hospital et British Columbia Women’s Hospital & Health Centre

Partners

The generosity of the partners plays a key role in the development and growth of the Sickle Cell Anemia Association of Quebec. Their support is invaluable. We thank them from the heart.